title: Importance of immune response genes in hemophilia A.
abstract: Hemophilia A is a disease caused by a deficiency of coagulation factor VIII resulting from genetic inheritance linked to chromosome X. One treatment option is the administration of plasma or recombinant FVIII. However, some patients develop inhibitors or antibodies against this factor. Inhibitors are alloantibodies that bind to the epitope of factor VIII causing it to be recognized by the immune system as a foreign peptide. This is the most serious complication in hemophilia patients in respect to replacement therapy. Some studies have suggested that genetic factors influence the development of factor VIII inhibitors such as ethnicity, family history, mutations in the factor VIII gene and in genes of the immune system. The aim of this study was to conduct a literature review to assess the influence of genetic factors of immune response genes, especially genes of the major histocompatibility complex and cytokines, which may be related to the development of factor VIII inhibitors in hemophilia A patients. Understanding these risk factors will help to determine future differential treatment in the control and prevention of the development of inhibitors.
authors.affiliation: Universidade Estadual de Maringá - UEM, Maringá, PR, Brazil.
authors.forename: Josiane Bazzo
authors.initials: JB
authors.lastname: de Alencar
authors.forename: Luciana Conci
authors.initials: LC
authors.lastname: Macedo
authors.forename: Morgana Ferreira
authors.initials: MF
authors.lastname: de Barros
authors.forename: Camila
authors.initials: C
authors.lastname: Rodrigues
authors.forename: Renata Campos
authors.initials: RC
authors.lastname: Cadide
authors.forename: Ana Maria
authors.initials: AM
authors.lastname: Sell
authors.forename: Jeane Eliete Laguila
authors.initials: JE
authors.lastname: Visentainer
keywords: Blood coagulation; Cytokines; Factor VIII; Hemophilia A; Human Leukocyte Antigen; Major histocompatibility complex